摘要
目的探讨儿童朗格汉斯细胞组织细胞增生症的临床特点。方法回顾性分析20例儿童朗格汉斯细胞组织细胞增生症患者的病例资料,总结临床和皮损表现特点。结果 20例患者首发有皮损11例(55.00%)、骨损害所致骨骼痛或皮下包块6例(30.00%)、淋巴结肿大2例(10.00%)、尿崩症1例(5.00%)。表现为单系统受累4例(20.00%),多系统受累16例(80.00%)(其中14例<2岁);风险器官受累11例(55.00%);死亡1例(5.00%)。15例(75.00%)患者有皮肤损害,均合并系统受累,躯干、头面部最常见。皮疹主要表现为丘疹、斑块、结节和脂溢性湿疹样损害两类。皮损发生年龄最早1个月,最晚11岁6个月,12例在1岁以前出现。结论朗格汉斯细胞组织细胞增生症皮肤表现多样,早期容易误诊。2岁以下儿童易发生多系统和风险器官受累,预后差。
Objective To investigate the clinical characteristics of Langerhans cell histocytosis（LCH）in children. Methods Retrospective analysis was performed on clinical data gathered from 20 pediatric patients with LCH. The clinical features and cutaneous presentations were summarized. Results The most common initial manifestations in the 20 patients were lesions（11/20, 55.00%）,followed by bone pain or subcutaneous mass（6/20, 30.00%）, lymphadenopathy（2/20, 10.0%）,and diabetes insipidus（1/20, 5.00%）. Among 20 patients,there were four cases（20.00%）with single system involvement,16 cases（80.00%）with multiply system involvement, in which 14 patients were younger than two years old,11 cases（55.00%）with risk of organ involvement and only one case（5.00%）dead. Of the 20 patients, 15 cases（75.00%）presented with skin manifestations accompanied by other organ involvement. The prominent sites were trunk,head and face. There were four kinds of cutaneous presentations as papule,plaque,nodule and seborrheic eczema-like lesions. The earliest and the latest onsets of skin manifestation were observed in patients one month and 11.5 years old, respectively. LCH was diagnosed in 16 patient younger than one year old.Conclusion LCH has variable skin manifestations and can be easily misdiagnosed in the early stage. Patients younger than 2 years age are more prone to have the multi-system and major organsinvolved, and they tend to have a less satisficed prognosis.
作者
李建红
李萍
唐鹏跃
罗珍
冯思航
刘建中
LI Jianhong;LI Ping;TANG Pengyue;LUO Zhen;FENG Sihang;LIU Jianzhong(Department of Dermatology, Shenzhen Children＇s Hospital, Shenzhen 518038, China)
出处
《中国皮肤性病学杂志》
CAS
CSCD
北大核心
2018年第6期650-653,共4页
The Chinese Journal of Dermatovenereology
